The acute hepatic porphyria is a rare inherited autosomal dominant dis
order of heme biosynthesis. It is caused by an enzyme defect of uropor
phyrinogen-l-synthestasis. The congenital disease is more often in fem
ale patients with a total prevalence of 5-10/100000. Medical and neuro
logic symptoms are common and porphyria is initially often misdiagnose
d. Psychiatric symptoms are depression, psychoorganic syndromes and sc
hizophrenic psychosis. In this article we describe psychopathological
symptoms reported in the literature and present a case of porphyria wi
th various psychopathological manifestations posing difficulties in di
fferentiating from other diagnosis.